Long-Term Effect of LIAM on Respiratory Performance in NIV Patients Suffering From Neuromuscular Disease

Neuromuscular Disease Clinical Trial

— LIAM  

Official title:

Long-Term Effect of LIAM (Lung Insufflation Assist Maneuver) on Respiratory Performance in Non-invasive Ventilated Patients Suffering From Neuromuscular Disease

Clinical Trial Details — Status: Recruiting

Administrative data

• NCT number: NCT02288299
• Other study ID #: 2014-A01005-42
• Status: Recruiting
• Phase: N/A
• Start date: October 29, 2015
• Est. completion date: December 31, 2021

Study information

• Verified date: February 2019
• Source: Centre d'Investigation Clinique et Technologique 805
• Contact: David Orlikowski, MD, PhD
• Phone: +33(0)147107777
• Email: david.orlikowski[@]rpc.aphp.fr
• Is FDA regulated: No
• Study type: Interventional

Clinical Trial Summary

The long-term effect of LIAM (Lung Insufflation Assist Maneuver) on respiratory performance in home non-invasively ventilated (NIV) patients suffering from neuromuscular disease will be assessed in a prospective, randomized, cross over, open label study

Description:

In advanced neuromuscular disorders, respiratory complications represent the main cause of morbidity and mortality. Beside chronic respiratory insufficiency, necessitating a ventilatory support, mostly performed by non-invasive ventilation (NIV), cough is impaired due to the muscle weakness, and respiratory physiotherapy becomes an essential part of the management.

Various techniques have been proposed to improve lung recruitment and cough in neuromuscular patients. The assisted techniques based on a positive pressure insufflation maneuver have shown an improvement in physiological variables on the short term, but there is to date no good-quality prospective study allowing to evaluate the long term efficacy of mechanical cough assistance devices in neuromuscular patients.

We designed a randomized, cross over, open label study to assess the long-term effect of LIAM (Lung Insufflation Assist Maneuver) on respiratory performance in NIV patients suffering from neuromuscular disease.

Recruitment information / eligibility

• Status: Recruiting
• Enrollment: 30
• Est. completion date: December 31, 2021
• Est. primary completion date: December 31, 2021
• Accepts healthy volunteers: No
• Gender: All
• Age group: 18 Years and older

Eligibility

Inclusion Criteria:

• o neuromuscular disease ( progressive muscular dystrophy, for example; Duchenne muscular dystrophy, spinal muscular atrophy)

• age = 18 years

• indication to NIV (ongoing ventilation or new patients)

• vital capacity = 50% pred

• peak cough flow < 270 l/min

• "LIAM Responder" = increased insufflation capacity (= + 50%) and Peak Cough Flow (PCF =+ 50%) with LIAM

Exclusion Criteria:

• o acute respiratory failure (respiratory acidosis)

• home treatment by instrumental cough assistance in the preceding 12 months

• ongoing medical treatment of the neuromuscular disease (for ex: corticosteroids in Duchenne disease, enzyme therapy in Pompe disease)

• previous pneumothorax

• plan of legal protection

• pregnant or breastfeeding women

• failure to cooperate

• no affiliation to a social security scheme

Related Conditions & MeSH terms

• Neuromuscular Disease
• Neuromuscular Diseases

Intervention

Device:
• non invasive mechanical ventilation
instrumental increase of inspiratory capacity and cough

Locations

Country	Name	City	State
France	Home ventilation Unit , Raymond Poincaré hospital	Garches	Paris Area

Sponsors (1)

Lead Sponsor	Collaborator
Centre d'Investigation Clinique et Technologique 805

Country where clinical trial is conducted

France, 

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	Change in the maximal assisted vital capacity (maximal insufflation capacity) between the begin of the treatment period and the 3-months follow up	Change in the maximal assisted vital capacity (maximal insufflation capacity) between the begin of the treatment period and the 3-months follow up	15 minutes
Secondary	spontaneous vital capacity	spontaneous vital capacity change at 3 months	15 minutes
Secondary	peak flow	peak cough flow (spontaneous / assisted) change at 3 months	15 minutes
Secondary	optoelectronic plethysmography	change at 3 months in the distribution in ventilation (assessed by optoelectronic plethysmography), spontaneous/assisted	1 hour